摘要目的:分析幼年局限性硬皮病(JLS)的临床表现、实验室检查特点及治疗转归。方法:回顾性病例系列研究。对2019年1月到2021年8月在首都医科大学附属北京儿童医院皮肤科诊治的JLS患儿的流行病学及临床资料进行分析。结果:228例患儿中男95例，女133例，男女比例为1.0∶1.4，发病中位年龄为67月龄(5.6岁)，从发病到诊断时间为1～106个月。8例(3.5%)患儿在发病前有可疑诱发因素，25例(11.0%)同时患有其他疾病，3例(1.3%)有自身免疫性疾病家族史。临床类型最常见的为局限性硬斑病(101例，44.3%)，其次为带状硬皮病(91例，39.9%)，泛发性硬皮病(11例，4.8%)，混合性硬皮病(23例，10.1%)，全硬化性硬皮病(2例，0.9%)。其中76例(46.3%)患儿抗核抗体(ANA)阳性，抗可溶性抗原抗体(ENA)阳性14例(15.0%)。91例局限性硬斑病患儿外用中强效糖皮质激素、维生素D3衍生物或钙调磷酸酶抑制剂治疗；其余类型患儿接受了系统糖皮质激素治疗，起始剂量1～2 mg/kg，最大剂量60 mg/d，其中72例联合甲氨蝶呤治疗，起始剂量10～15 mg/m 2，每周1次；9例联合生物制剂治疗。随访结果显示在皮肤科门诊随诊的患儿皮肤症状有一定程度的改善，能够保持处在疾病不活动状态。 结论:在皮肤科就诊的JLS患儿以学龄前期及学龄期儿童为主；局限性硬斑病最常见，以外用糖皮质类固醇、维生素D3衍生物或钙调磷酸酶抑制剂为主；无特异性实验室检查指标；其他类型JLS系统治疗推荐糖皮质激素联合甲氨蝶呤。

abstractsObjective:To analyze the clinical manifestations, laboratory results and treatment outcomes of juvenile localized scleroderma(JLS).Methods:This was a retrospective case series study.Epidemiological and clinical data of patients with JLS treated in the Department of Dermatology, Beijing Children′s Hospital, Capital Medical University from January 2019 to August 2021 were analyzed.Results:Among the 228 children, there were 95 males and 133 females, with a male-to-female ratio of 1.0∶1.4.The median age of onset was 67 months(5.6 years), and the time from onset to diagnosis was 1 month to 106 months.Eight patients(3.5%) had suspected predisposing factors before onset, 25 patients(11.0%) had other diseases at the same time, and 3 patients(1.3%) had a family history of autoimmune diseases.The most common clinical type of JLS was circumscribed morphea(101 cases, 44.3%), followed by linear scleroderma(91 cases, 39.9%), generalized morphea(31 cases, 13.6%), mixed scleroderma(23 cases, 10.1%) and pansclerotic morphea(2 cases, 0.9%).Among them, 76 patients(46.3%) were antinuclear antibody(ANA) positive, and 14 patients(15.0%) were extractable nuclear antigen antibody(ENA) positive.Ninety-one of patients with circumscribed morphea were treated with medium and strong corticosteroids, vitamin D3 derivatives or calcineurin inhibitors.Patients of other types received systemic corticosteroid treatment, with an initial dose range of 1-2 mg/kg and a maximum dose of 60 mg/d.Among them, 72 patients were additionally treated with Methotrexate, with an initial dose range of 10-15 mg/m 2, once a week, and 9 patients were additionally treated with biological agents.The follow-up results showed that the skin symptoms of the patients who were followed up in the dermatology outpatient department had improved to a certain extent and could remain inactive. Conclusions:Children with JLS in the dermatology department are mainly preschool- and school-age.Circumscribed morphea is the most common type, mainly treated with glucocorticoids, vitamin D3 derivatives or calcineurin inhibitors.No specific laboratory test index is found.Corticosteroids combined with Methotrexate are recommended for systematic treatment of other types of JLS.